Download Pain management in Ehlers Danlos Syndrome

Pain management in Ehlers Danlos Syndrome
Pradeep Chopra, MD, MHCM
Director, Pain Management Center, Assistant Professor, Brown Medical School, Rhode Island
Assistant Professor (Adjunct), Boston University Medical Center
[email protected]
[email protected]
Pradeep Chopra, MD
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Disclosure and disclaimer
• I have no actual or potential conflict of interest in relation to this presentation or program
• This presentation will discuss “off‐label” uses of medications
• Discussions in this presentation are for a general information purposes only. Please discuss with your physician your own particular treatment. This presentation or discussion is NOT meant to take the place of your doctor. Pradeep Chopra, MD
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Introduction
• Training and Fellowship, Harvard Medical school
• Pain Medicine specialist
• Assistant Professor – Brown Medical School, Rhode Island
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Pain in EDS by body parts
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Head and neck Shoulders
Jaws
Chest
Abdomen
Hips
Lower back
Legs
Complex Regional Pain Syndrome – CRPS or RSD
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Pain in EDS
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From nerves – neuropathic
From muscles – Myofascial From Joints – nociceptive pain
Headaches
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Muscle pain
Myofascial pain
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Muscle Pain
• Muscles are held together by fascia – ‘saran wrap’ which is made of collagen
• Muscle spasms or muscle knots develop to compensate for unbalanced forces from the joints
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Muscle pain 1
• Most chronic pain conditions are associated with muscle spasms
• Often more painful than the original pain
• Muscles may tighten reflexively, guarding of a painful area, nerve irritation or generalized tension
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Muscle pain 2
• Myofascial pain syndrome is a chronic form of muscle pain. • Myofascial pain syndrome centers around sensitive points in your muscles called trigger points
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Treatment of muscle pain
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Treat the cause – joint pain, repetitive use Trigger point injections
Stretching Relaxation techniques
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Trigger Points
• When muscles are in chronic spasm they stay taut – ‘knots’. • They develop trigger points which trigger the muscle in to a constant state of spasm
• The trigger points can be painful when touched. • The pain can spread throughout the affected muscle and other parts
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Manual Techniques
• Myofascial release: Gentle techniques to improve the mobility of the muscles by mobilizing the tissue around the muscle
• Muscle energy technique: Utilizes positioning of the body and using active muscle contraction to relax muscles and align joints
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Manual Therapy Techniques
• Strain‐Counterstrain: A positional technique to help relax tight muscles
• Craniosacral Therapy: A gentle technique to help balance the nervous system
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Muscle Relaxants
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Cyclobenzaprine (Flexeril)
Carisoprodol (Soma)
Tizanidine (Zanaflex)
Baclofen
Benzodiazipine (Valium)
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Muscle relaxants
• Not true muscle relaxants
• Decrease tone
• Lethargy – good for bedtime dosing to sleep
• Approved for long term use: Baclofen, tizanidine
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Risk Factors
• Muscle injury. Stress on your muscles can cause trigger points to form.. Repetitive stress also may increase your risk.
• Inactivity. If you've been unable to use a muscle, such as after surgery or after a stroke, you may experience trigger points in your muscle as you start to move it during your recovery.
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Nerve pain
Neuropathic pain
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Nerve pain in Connective tissue disorder / EDS
• Nerve connective tissue is more fragile
• Fragile nerves get over stretched when crossing or associated with hypermobile joints
• Small Fiber neuropathy
• Neuropathic pain, Complex Regional Pain Syndrome (CRPS) or Reflex Sympathetic Dystrophy (RSD)
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Nerve pain in EDS ‐ Mitochondria
• Is it Mitochondrial disorder ? • Mitochondria found in cells of the human body
• They produce energy • Suspect, if the pain is widespread and involves nerves
• Exercise intolerance, muscle weakness, seizures, developmental delays etc. Pradeep Chopra, MD
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Neuropathic pain medications
• Gabapentin (Neurontin®)
• Pregabalin (Lyrica®)
• Duloxetine (Cymbalta®)
• Milnacipran (Savella®)
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Gabapentin and pregabalin
• Promising results have been shown.
• Gabapentin – go up to 900mg, then 1800mg. Slow acting drug. • Pregabalin – 150mg to 300mg. Faster acting drug. • The difference may not be obvious at first but when they come off it, they notice an increase in pain
• Chance of increased dizziness in POTS ‐ usually stops after being on the drug for sometime. • Start low, go slow
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SNRI (selective nor‐epinephrine release inhibitors)
• Duloxetine (Cymbalta®)
• Milnacipran (Savella®)
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SNRI (selective nor‐epinephrine release inhibitors)
• Increase nor ‐ epinephrine levels in the central nervous system
• Increased nor‐epinephrine levels help in modulating pain signals
• Milnacipran increases nor‐epinephrine by 3 times as compared to duloxetne
• May increase blood pressure and heart rate Pradeep Chopra, MD
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Antidepressants
• Tricyclic antidepressants (TCA) well studied in neuropathic pain • Reuptake blockers of serotonin and noradrenaline (Amitrityline, nortriptyline) –
work well
• SSRI (Prozac, Zoloft) – do not work well for pain
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Reflex Sympathetic Dystrophy (RSD)
Complex Regional Pain Syndrome (CRPS)
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Common clinical features of RSD
• Continuous burning pain
• Pain disproportionate in intensity to the inciting event
• Pain to touch – Allodynia
• Pain not in any specific nerve distribution or even to the site of injury
• Swelling
• Increased / decreased sweating
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Symptoms of CRPS / RSD
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Pain – severe, constant
Temperature difference
Hypersensitivity Tremor, Involuntary movements, muscle spasms, atrophy (weakening of the muscle)
Increased sweating
Color difference
Bone thinning
Swelling
Hair and nail changes
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Headaches and Neck pain
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Headaches
• Mostly unknown cause
• Cerebrovascular reactivity • Prone to migraines and Tension Type Headaches
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Headaches and neck pain
• Headaches may be caused by neck pain
• Headaches may be from – inside the head (Migraines) or – outside (chronic daily headaches or tension type headaches)
• Treatment for both is different
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Headaches and Neck pain
• C1‐C2 instability
• Kyphosis (Bent backwards) of neck spine
• Degeneration of neck disks (especially C4‐C5, C5‐C6)
• Cranio‐cervical instability (junction between head and neck not stable)
• Chiari malformation
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Symptoms of neck (C1‐C2) instability
• Neck pain • Headaches in the back of the head (occipital headaches)
• Fainting sensation with rotating head to the side
• Choking sensation
• Treatment – surgical fusion, strengthening of neck muscles, try bracing for flare ups
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Migraines
• Treatment depends on the frequency – if they are occasional, do not treat
• Abortives for infrequent migraines
• Prophylactic for frequent migraines
• Triptans (Rizatriptan ‐ Maxalt®, Sumatriptan ‐ Imitrex®, etc – very effective as abortive, not prophylactic)
• Triptans not approved for children under 12 years • Prophylactic in children – Cyprohetadine ‐ Periactin® Improves appetite also
• Other prophylactics – amitriptyline, nortriptyline, topiramate
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Red Flags
• Acute (sudden) abdominal or flank pain may be due to a ruptured uterine, intestine or twisted intestine
• Bleeding in the head may present with sharp pain in head, change in mental status or seizure
• Emergency
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TMJ Pain
Temporo Mandibular Joint Dysfunction
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TM Joint pain Pradeep Chopra, MD
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Temporo‐mandibular joint dysfunction (TMJ) Present in 70%
Clicking sound
Grinding at night or clenching teeth
Joint pain, chewing muscle pain
Treatment: avoid excessive mouth opening, caution when yawning, orthodontist specializing in TMJ • Avoid over the counter mouth guards
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Arm and leg pain
• Brachial plexus neuropathy
• Lumbosacral neuropathy
• Mechanical pressure due to dislocations and subluxations
• Nerve disorders in EDS make them prone to damage
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Spine pain
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Facet joint instability
Sacroiliac joint pain
Muscles around the spine
Degenerative disc disease
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Spine pain
• Steroid injections • Core strengthening exercises
• Temporary brace if flare up
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Tendons and Ligaments
• Ligaments and tendons are made of connective tissue
• Ligaments connect bone to bone
• Tendons connect muscle to bone
• Tendons are an extension of the strong connective tissue that surrounds all muscles – the fascia
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Tendon and ligament pain
• Bracing or spica
• Topicals
• Preventive measures
– Avoid overstretching or hyperextending joints – Avoid high impact activities
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Prevention
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Preventing pain
• Do not stand on one hip or sway at hips
• Do not stand on the outside of your feet
• Do not sleep on your stomach, head turned to one side for a prolonged period
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Preventing pain
• Do not sit with legs outstretched, or with legs tucked under the buttocks ‘W’ position
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Preventing pain
• Do not hyperextend knees when standing
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Preventing pain
• Do not sit kneeling with buttocks resting on ankles
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Preventing Pain – working at home
• Avoid repetitive activities – vacuuming, raking, filling dish washer, stirring. Change activity frequently. • Work surface (kitchen counter, sink etc.) should be at appropriate height. Use blocks to raise your work surface
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Preventing pain ‐ sleeping
• EDS patients wake up with pain and stiffness
• Use a soft mattress
• Prefer using separate mattresses for partner and yourself
• Feather pillow or memory foam pillows
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Preventing pain ‐ shopping
• Do not carry a heavy or light bag hanging from a shoulder
• Do not carry grocery bags in hand, especially heavy one. Keep them light. • Use shopping trolley. Use wheels wherever you can
• Keep handbags light
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Preventing pain – clothes
• Keep clothes loose and light
• Women – bra with wide straps and possibly cross‐over straps
• Wear light and soft shoes. Use well balanced orthotics
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Preventing pain – going to the dentist
• EDS’ers wind up making more trips to the dentist
• Talk to dentist about EDS
• Numbing medicine may not work or you maybe too sensitive to them
• TMJ – not to keep mouth open too long
• Position of neck – prefer being in a more flat position with neck supported
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Medications
• Opioid or narcotics
• NSAID’s (non steroidal anti‐inflammatory drugs)
• Acetaminophen / paracetamol
• Anti‐depressants
• Anticonvulsants
• Other
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LDN
Low Dose Naltrexone
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Low Dose Naltrexone (LDN)
• Naltrexone blocks the effect of opioids. • Approved 30 years ago by the FDA for opioid and alcohol addiction
• In the late 1980’s it was discovered that at low doses it helped patients with immune disorders. • Since then, LDN has been used extensively by patients with immune disorders like Multiple sclerosis, Crohn’s disease, Rheumatoid arthritis
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Low Dose Naltrexone (LDN)
• There are several theories as to how LDN may work
• Low side effect profile
• Inexpensive
• Useful for chronic pain • Useful for symptoms of EDS
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Low Dose Naltrexone (LDN)
• Good experience with using in EDS and chronic pain
• Unsure as to how it helps EDS. • Most patients with EDS report improvement in function Pradeep Chopra, MD
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Acetaminophen / Paracetamol
• One of the safest analgesics
• By itself is good for mild to moderate pain
• Potentiated effect when combined with other analgesics such as NSAIDS (Naproxen, Ibuprofen) or opioids (Codeine, Oxycodone). • Potentiating effect helps lower the need to take NSAIDs and Opioids. • Keep an eye on maximum dose – 3000gms/ day ( 9 regular Tylenols®)
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NSAID (Non steroidal anti‐
inflammatory drugs)
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Naproxen, Ibuprofen etc
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Good analgesics. Better if combined with acetaminophen/paracetamol
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Significant side effects – gastric irritation, kidneys
Avoid in Irritable Bowel Syndrome, Ulcerative Colitis
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Not useful in neuropathic pain •
May be used in joint pain •
Better off using it as a topical for superficial joints (Shoulder, knee, ankles, hands)
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Topicals
• NSAID’s are commonly effective
• Avoids the side effects of oral medicines • Far more effective locally at the site of pain
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Topicals – ointment, lotions, patches
• Compounding pharmacies can make a compound with NSAID’s
• DMSO and Active Max® – use as additive to help better peneration
• Diclofenic acid with DMSO (Pennsaid®)
• Diclofenac acid (Voltaren® gel)
• Flector® patch helpful
• Lidocaine patch not helpful
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Opioids
• Counterproductive for chronic pain
• Mild doses for a short term are good for acute pain
• Safer than using NSAID’s (ibuprofen, naproxen)
• Maybe combined with NSAID’s or acetaminophen for better effect and lower dose
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Prevalence of Addiction
Total Pain
Population
Addiction: 2% to 5%
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Webster LR, Webster RM. Pain Med. 2005;6(6):432-442.
Gluten free diet
• Gluten is a protein found in wheat, rye, barley and other grains
• One can develop an intolerance at any age. • Gluten as a protein can cause an inflammatory response in the body.
• Migraines, chronic body ache, abdominal pain, Fibromyalgia, hypermobility syndromes (EDS), multiple joint pains
• Hold off on gluten foods for 8 weeks to see if it makes a difference. Pradeep Chopra, MD
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Starting treatment ‐
medicines and exercise
Start low, go slow
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Service Dogs
• Trained to each person’s physical impairments
• help with functioning and independence
• Constant companion, will often sense its owners pain and will comfort them both physically and emotionally
• Can sense distress and call for help
• Service dogs give patients a feeling of security allowing them to be more active physically and socially • Provide stability while walking, open and close doors, switch on and off lights
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Service Dogs
• POTS – they can sense when their owner is having an episode of dizziness or seizure
• EDS and pain ‐ they protect the limb from being injured or touched
• Helps boost confidence in their owners. Pradeep Chopra, MD
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Watson and Usher
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Exercise and Physical Therapy
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EDS – Exercise and Physical Therapy Goals
• Restore function
• Learn how to properly adjust limb movements
• Muscle strengthening
• Aqua therapy
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Physical Therapy ‐ two types
• Patients who have recently developed pain –
PT should focus more on pain
• Patients who have had for a while (Chronic) –
PT should be more time based
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Physical Therapy – Pain focused
• The level and progression of exercise is determined by the intensity of the pain. • The intensity of the exercise should be low in cases of (or days of) highly intense pain. • Similarly, the intensity of exercise should be high if the pain is relatively low. • Its not harmful if there is an increase in pain for a short duration (approx. 1 to 2 hours) after exercise
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Physical Therapy – Pain Focused
• A program is designed based on the patients limitations • Patient exercise within those limits. • It builds confidence and increases confidence in using their limbs
• Pain focused PT has been shown to decrease considerably symptoms of recent onset RSD
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Physical Therapy – Time focused
• The level of exercise is built over time unrelated to intensity of pain
• Preferred approach in cases of long standing
• Pain focused PT can give way to Time Focused PT and vice versa
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